CPOX Gene Summary [Human]

The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).[provided by RefSeq, Oct 2009]

Details

Type
Protein Coding
Official Symbol
CPOX
Official Name
coproporphyrinogen oxidase [Source:HGNC Symbol;Acc:HGNC:2321]
Ensembl ID
ENSG00000080819
Bio databases IDs NCBI: 1371 Ensembl: ENSG00000080819
Aliases coproporphyrinogen oxidase, coproporphyria, homozygous coproporphyria
Synonyms cac, Coprogen oxidase, coproporphyrinogen oxidase, COX, CPO, CPX, HARPO, HCP, HEM6, M100835, nct, Rgsc835
Species
Human, Homo sapiens
OrthologiesMouseRat

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in human CPOX often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • structural constituent of eye lens
  • coproporphyrinogen oxidase
  • protein homodimerization
  • Na+/K+-exchanging ATPase
  • enzyme
  • identical protein binding
  • Coproporphyrinogen III oxidase

Pathways

Biological processes and signaling networks where the CPOX gene in human plays a role, providing insight into its function and relevance in health or disease.

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
  • hereditary disorder
  • coproporphyria
  • harderoporphyria
  • organismal death
  • dominant hereditary coproporphyria
  • digenic coproporphyria
regulated by
regulates
  • heme
  • Na+
  • K+
  • protoporphyrinogen
  • heme a

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Cytoplasm
  • cellular membrane
  • Mitochondria
  • cytosol
  • mitochondrial intermembrane space
  • mitochondrial inner membrane

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the human CPOX gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • heme O biosynthetic process
  • heme biosynthetic process
  • heme a biosynthetic process
  • protoporphyrinogen IX biosynthetic process
  • heme b biosynthetic process

Cellular Component

Where in the cell the gene product is active
  • mitochondrial intermembrane space
  • cytoplasm
  • mitochondrion
  • membrane
  • cytosol

Molecular Function

What the gene product does at the molecular level
  • protein homodimerization activity
  • coproporphyrinogen oxidase activity
  • structural constituent of eye lens

Gene-Specific Assays for Results You Can Trust

Streamline your workflow with assays designed for this gene. Our targeted dPCR and qPCR assays help you generate meaningful data – efficiently and accurately.